Muscle Biopsy Findings in Combination With Myositis-Specific Autoantibodies Aid Prediction of Outcomes in Juvenile Dermatomyositis.
Muscle Biopsy Findings in Combination With Myositis-Specific Autoantibodies Aid Prediction of Outcomes in Juvenile Dermatomyositis.
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DOI：
10.1002/art.39753
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发表时间：
2016-11
影响因子：
13.3
通讯作者：
Wedderburn, Lucy R.
中科院分区：
文献类型：
--
作者：
Deakin, Claire T.;Yasin, Shireena A.;Simou, Stefania;Arnold, Katie A.;Tansley, Sarah L.;Betteridge, Zoe E.;McHugh, Neil J.;Varsani, Hemlata;Holton, Janice L.;Jacques, Thomas S.;Pilkington, Clarissa A.;Nistala, Kiran;Wedderburn, Lucy R.
青少年皮肌炎(DM)是一种罕见而严重的自身免疫性疾病，以皮疹和近端肌肉无力为特征。虽然一些患者对标准治疗有反应，但另一些患者则没有。本研究旨在探讨青少年DM的组织病理学表现和肌炎特异性自身抗体(MSA)是否对预后有意义。来自英国青少年皮肌炎队列和生物标记物研究患者的肌肉活检样本(n = 101)被染色、分析并对组织病理学特征的严重程度进行评分。此外，还检测了患者(n = 90)的血清或血浆中的自身抗体，并收集了纵向临床数据(中位随访时间为4.9年)。长期治疗状态(随着时间的推移接受或停止药物治疗)使用广义估计方程进行建模。肌肉活检评分根据MSA亚组不同而不同。当考虑到MSA亚组的影响时，肌肉组织病理学特征严重程度的增加预示着随着时间的推移继续接受治疗的风险增加：对于全球病理评分(组织病理学专家的视觉模拟评分[HVAS]评分)，比数高1.48倍(95%可信区间[95%CI]1.12-1.96；P = 0.0058)，对于总的活检评分(用标准评分工具确定)，比数高1.10倍(95%CI1.01-1.21；P = 0.038)。在有抗Mi-2自身抗体的患者中发现了保护作用，尽管肌肉活检评分显示疾病更严重，但他们继续治疗的几率降低了7.06%(95%CI1.41-35.36；P = 0.018)。在抗核基质蛋白2自身抗体、抗转录中介因子1γ自身抗体或未检测到自身抗体的患者中，在不考虑MSA亚型的影响的情况下，单独增加组织病理学严重程度可以预测继续治疗的风险：对于HVAS总体病理评分，OR是1.61倍(95%CI1.16~2.22；P =0.004)，对于总活检评分，OR是1.13倍(95%CI1.03~1.24；P = 0.013)。组织病理学严重程度结合MSA亚型可预测幼年型糖尿病患者继续治疗的风险，并可能有助于与父母和患者讨论可能的治疗时间。了解这些关联可能会识别出患严重疾病的风险更高的患者。
Juvenile dermatomyositis (DM) is a rare and severe autoimmune condition characterized by rash and proximal muscle weakness. While some patients respond to standard treatment, others do not. This study was carried out to investigate whether histopathologic findings and myositis‐specific autoantibodies (MSAs) have prognostic significance in juvenile DM. Muscle biopsy samples (n = 101) from patients in the UK Juvenile Dermatomyositis Cohort and Biomarker Study were stained, analyzed, and scored for severity of histopathologic features. In addition, autoantibodies were measured in the serum or plasma of patients (n = 90) and longitudinal clinical data were collected (median duration of follow‐up 4.9 years). Long‐term treatment status (on or off medication over time) was modeled using generalized estimating equations. Muscle biopsy scores differed according to MSA subgroup. When the effects of MSA subgroup were accounted for, increased severity of muscle histopathologic features was predictive of an increased risk of remaining on treatment over time: for the global pathology score (histopathologist's visual analog scale [hVAS] score), 1.48‐fold higher odds (95% confidence interval [95% CI] 1.12–1.96; P = 0.0058), and for the total biopsy score (determined with the standardized score tool), 1.10‐fold higher odds (95% CI 1.01–1.21; P = 0.038). A protective effect was identified in patients with anti–Mi‐2 autoantibodies, in whom the odds of remaining on treatment were 7.06‐fold lower (95% CI 1.41–35.36; P = 0.018) despite muscle biopsy scores indicating more severe disease. In patients with anti–nuclear matrix protein 2 autoantibodies, anti–transcription intermediary factor 1γ autoantibodies, or no detectable autoantibody, increased histopathologic severity alone, without adjustment for the effect of MSA subtype, was predictive of the risk of remaining on treatment: for the hVAS global pathology score, 1.61‐fold higher odds (95% CI 1.16–2.22; P = 0.004), and for the total biopsy score, 1.13‐fold higher odds (95% CI 1.03–1.24; P = 0.013). Histopathologic severity, in combination with MSA subtype, is predictive of the risk of remaining on treatment in patients with juvenile DM and may be useful for discussing probable treatment length with parents and patients. Understanding these associations may identify patients at greater risk of severe disease.
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影响因子：
6.3
作者：
Twisk, JWR;Smidt, N;de Vente, W
通讯作者：
de Vente, W
影响因子：
4.7
作者：
Rider, Lisa G.;Koziol, Deloris;Giannini, Edward H.;Jain, Minal S.;Smith, Michaele R.;Whitney-Mahoney, Kristi;Feldman, Brian M.;Wright, Susan J.;Lindsley, Carol B.;Pachman, Lauren M.;Villalba, Maria L.;Lovell, Daniel J.;Bowyer, Suzanne L.;Plotz, Paul H.;Miller, Frederick W.;Hicks, Jeanne E.
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Hicks, Jeanne E.
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5.5
作者：
Martin, Neil;Krol, Petra;Wedderburn, Lucy R.
通讯作者：
Wedderburn, Lucy R.
影响因子：
4.7
作者：
Huber, Adam M.;Giannini, Edward H.;Bowyer, Suzanne L.;Kim, Susan;Lang, Bianca;Lindsley, Carol B.;Pachman, Lauren M.;Pilkington, Clarissa;Reed, Ann M.;Rennebohm, Robert M.;Rider, Lisa G.;Wallace, Carol A.;Feldman, Brian M.
通讯作者：
Feldman, Brian M.
影响因子：
27.4
作者：
Varsani H;Charman SC;Li CK;Marie SK;Amato AA;Banwell B;Bove KE;Corse AM;Emslie-Smith AM;Jacques TS;Lundberg IE;Minetti C;Nennesmo I;Rushing EJ;Sallum AM;Sewry C;Pilkington CA;Holton JL;Wedderburn LR;UK Juvenile Dermatomyositis Research Group
通讯作者：
UK Juvenile Dermatomyositis Research Group